Endovascular rescue of vertebro-basilar thrombosis in cervical spine injury.

INTRODUCTION: Vertebro-basilar thrombosis is often lethal. We report a post-traumatic case in which logistical issues were overcome to secure prompt endovascular intervention resulting in a favourable outcome. CASE PRESENTATION: We report this case to highlight (i) the need, across the United Kingdom, for rapid access to 24/7 neurointerventional services and (ii) the fact that vertebral artery injury during C-spine trauma, while not uncommon, can rarely have catastrophic complications. DISCUSSION: Vertebral artery injuries during C-spine trauma can rarely present as a neurovascular emergency requiring neurointerventional rescue. It is our opinion that patients with neurovascular emergencies merit 24/7 neurointerventional services, akin to those provided for acute coronary syndromes.

First demonstration of resolution of an infundibulum by direct treatment of the arterial wall with Pipeline flow-diverting stent.

INTRODUCTION: Infundibula are symmetrical funnel-shaped widening, measuring more than 3 mm at its base with the branch artery arising from its apex. The pathological significance of this entity remains controversial. Although a relatively common appearance in otherwise normal angiograms, they have occasionally been described to progress into saccular aneurysms or directly rupture. METHODS/RESULTS: We describe the first case of a disappearing infundibulum after a flow diverting stent was deployed across the infundibulum during the treatment of an adjacent aneurysm. CONCLUSIONS: We concur in the view that infundibula are at least pre-pathological lesions that may in certain circumstances require consideration for treatment. Our case for the first time offers a potential treatment option for such situations.

Spontaneous resolution of a colloid cyst of the third ventricle.

We report a case of an asymptomatic colloid cyst of the third ventricle in a 35-year-old male, which on follow-up MRI at 15 months appears to have spontaneously resolved. To our knowledge, this is the first such case reported and supports the role of conservative management of small asymptomatic colloid cysts.

A rete mirabile of the posterior cerebral artery. Embryonic and phylogenetic remnant of choroidal artery origin.

SUMMARY: We present the case of a 41-year-old man with a vascular network associated with the left posterior cerebral artery (PCA). Although initially considered to be a brain AVM, further investigation showed no arteriovenous shunting and a rete mirabile of the PCA was diagnosed. A well known but rare occurrence mainly in the anterior circulation, rete mirabile has not previously been described in the PCA.The PCA is a composite vessel, developmentally. Its proximal portion derives from the posterior division of the ICA while its cortical territories are annexed from the anterior choroidal artery adjacent to the lateral geniculate body. We propose that this rete (or network) represents a persistence of the embryonic anastomosis betweenthe AChA and the PCA.

Differences between intracranial vascular malformation types in the characteristics of their presenting haemorrhages: prospective, population-based study.

OBJECTIVE: To determine the imaging and demographic characteristics of intracranial haemorrhages, which are subsequently found to be due to an underlying intracranial vascular malformation (IVM). METHODS: We compared the demographic and brain imaging characteristics of adults presenting with intracranial haemorrhage, subsequently found to be due to a brain arteriovenous malformation (BAVM), dural arteriovenous fistula (DAVF) or cavernous malformation (CM) in a prospective, population-based cohort of adults diagnosed for the first time with an IVM (The Scottish IVM Study (SIVMS)). RESULTS: Of the 141 adults in SIVMS who presented with intracranial haemorrhage, those with CMs presented at a younger age and were less handicapped. A total of 115 (82%) had intracerebral haemorrhage (ICH) with or without subarachnoid, intraventricular or subdural extension. ICH without extension into other compartments accounted for all CM bleeds, but only 50% of BAVM and DAVF bleeds. Median haematoma volumes differed (Kruskal-Wallis, p<0.0001): ICH due to BAVM (16.0 cm3, inter-quartile range (IQR) 4.7 to 42.0) and DAVF (14.1 cm3, IQR 4.9 to 21.5) were similar, but CM haematoma volumes were smaller (median 1.8 cm3, IQR 1.3 to 4.3). These findings were robust in sensitivity analyses. Small haematoma volumes occurred among all IVM types; the largest haematoma volume due to CM was 12 cm3, and volumes of >34 cm3 were only due to BAVM. CONCLUSIONS: Intracranial haemorrhages found to be due to IVMs differ in adults' age of presentation and clinical severity, as well as the volume and distribution of the haematoma within the brain compartments.

Segmental neurovascular syndromes in children.

The concept of segmental vascular syndromes with different, seemingly unrelated, diseases is based on the embryology of the neural crest and the mesoderm migration of cells that share the same metameric origin. Migrating patterns of these cells link the brain, the cranial bones, and the face on the same side. A somatic mutation developing in the region of the neural crest or the adjacent cephalic mesoderm before migration can, therefore, be postulated to produce arterial or venous metameric syndromes, including PHACES, CAMS, Cobb syndrome, and Sturge-Weber syndrome. Although these diseases may be rare, their relationships among each other and their postulated linkage with the development of the neural crest and the cephalic mesoderm may shed light on the complex pathology and etiology of various cerebral vascular disorders.

Terminal triplication of the left vertebral artery: a unique variation.

SUMMARY: This report describes a unique case of triplication of the terminal left vertebral artery, forming the basilar artery in a 75-year-old male. CT angiography of cranio-cervical vessels also demonstrated the right vertebral artery originating from the right common carotid and an aberrant right subclavian artery.To the best of our knowledge this is the first report of a variation of this nature. The embryology and the clinical importance are discussed.

PHACES syndrome: a review of eight previously unreported cases with late arterial occlusions.

PHACE and PHACES are acronyms for a syndrome of variable expression comprising posterior cranial fossa malformations, facial haemangiomas, arterial anomalies, aortic coarctation and other cardiac disorders, ocular abnormalities and stenotic arterial disease. We review five girls and three boys aged 1 month-14 years with disorders from this spectrum. Six had large facial haemangiomas but recent reports suggest that small haemangiomas may occur; hence our inclusion of two possible cases. We also focus on the recently recognised feature of progressive intracranial arterial occlusions, present in four of our patients, later than previously recognised, from 4 to 14 years of age. We suggest that many elements of this disorder could reflect an abnormality of cell proliferation and apoptosis.

Mid-line clefts of the atlas: a diagnostic dilemma.

Congenital clefts and other developmental anomalies of the atlas vertebra are rarely encountered. They are incidental findings discovered while investigating the cervical spine following trauma. Differentiation of developmental variants of the atlas from the burst fracture of Jefferson is essential to prevent unnecessary medical intervention.

Intracranial haemorrhage within the first two years of life.

BACKGROUND: Spontaneous intracranial haemorrhage is not common in infants, with differences from adults in both aetiology and severity. The infantile CNS is more vulnerable because of incomplete hydrovenous maturation. We analyzed infantile intracranial haemorrhage mainly caused by structural brain lesions and discuss specific aetiologies with regard to haemodynamic characteristics. SUBJECTS AND METHODS: We reviewed 20 infants less than 2 years of age from a total of 328 neonates and infants with intracranial vascular lesions seen in our institution since 1985. Associated or causative lesions were arteriovenous malformation (AVM) in 6, dural sinus malformation (DSM) in 4, arteriovenous fistula (AVF) in 3, aneurysm in 2, developmental venous anomaly (DVA) in 1, vein of Galen malformation (VGAM) in 1, and others in 3. The locations of haematomas were intracerebral (ICH) in 8, combined ICH and intraventricular haemorrhage (IVH) in 5, IVH alone in 5, subarachnoid haemorrhage (SAH) in 1, and combined SDH and ICH in 1. FINDINGS: Three patterns of haemorrhage were noted in high-flow vascular lesions such as AVM or AVF (n=9); haemorrhage at the site of nidus or fistula corresponding to nidal pseudoaneurysm in 4, regional venous hypertension with pial venous reflux in 3, global venous infarction causing multifocal haemorrhage in distant brain areas in 2. Aneurysmal bleeds were caused by dissecting aneurysms at the level of dural penetration of cranial vessels. One infant had haemorrhage near a DVA without evidence of cavernous malformation suggesting the possibility of venous ischaemia. IVH was associated with shunt operations in 4 infants with DSM, and SDH followed by ICH in a infant with VGAM. INTERPRETATION: Spontaneous intracranial haemorrhage in infants and neonates is rare; it is associated with specific lesions which show some differences from their adult counterparts. The vein-related causes of hemorrhage are largely the pathophysiologic characteristics in this age group. Absence of hemorrhage in VGAM is remarkable in addition to occurrence of most hemorrhages after shunting.

Wyburn-Mason or Bonnet-Dechaume-Blanc as Cerebrofacial Arteriovenous Metameric Syndromes (CAMS). A New Concept and a New Classification.

SUMMARY: The diagnosis of Bonnet-Dechaume-Blanc or Wyburn-Mason syndrome encompasses a spectrum of phenotypic expression. Features of the syndrome as originally described, and common to all, include arteriovenous malformations of the brain and orbit (with retinal and/or retrobulbar lesions). A portion of these patients manifest the complete expression of the disease with additional high-flow arteriovenous malformations of the maxillofacial or mandibular regions. These present the distinct and additional risks of lifethreatening epistaxis or gingival haemorrhage. We suggest new diagnostic criteria for the syndrome. Applying insights from modern developmental biology to our series of 15 patients (the largest to date), together with a review of the literature, we have recognised metameric patterns of involvement in what we believe to be a disease of the neural crest or adjacent cephalic mesoderm. This allows us to propose a new rational classification reflecting the putative, underlying disorder and to suggest a new name: Cerebrofacial Arteriovenous Metameric Syndrome (CAMS).

Radiologists and visual acuity.

Visual acuity (VA) and potential risks to the radiologist's eyesight have been relatively neglected subjects in the radiological literature. This study comprises two parts, the first consisting of a questionnaire on this subject sent to a random sample of 480 practising radiologists in the United Kingdom, and the second, a spot check of the VA of radiologists in our department. Of questionnaires, 73 %were returned. Of respondents, 76 % felt that ionising radiation could affect their vision, but only 13 % used lead glasses on a regular basis. A total of 71 % felt that regular monitoring of eyesight should be required. Of 25 tested radiologists, 5 had suboptimal VA and could benefit from further correction. The pertinent literature is reviewed, and a case for periodic eyesight testing is presented, including VA and grey-scale discrimination.